• Franziska Köhler, Niels Matthes, Mathias Rosenfeldt, Volker Kunzmann, Christoph-Thomas Germer, and Armin Wiegering.
• Department of General, Visceral, Transplant, Vascular and Pediatric Surgery, University Hospital of Würzburg, Germany; Comprehensive Cancer Center Mainfranken, University Hospital of Würzburg, Germany; Institute of Pathology, University of Würzburg, Germany; Medical Clinic and Polyclinic II, University Hospital of Würzburg, Germany; Department of Biochemistry and Molecular Biology, University of Würzburg, Germany.
• Dtsch Arztebl Int. 2023 Aug 7; 120 (31-32): 519525519-525.

BackgroundNeoplasms of the vermiform appendix are rare. They comprise a heterogeneous group of entities requiring differentkinds of treatment.MethodsThis review is based on publications retrieved by a selective literature search in the PubMed, Embase, and Cochranedatabases.Results0.5% of all tumors of the gastrointestinal tract arise in the appendix. Their treatment depends on their histopathologicalclassification and tumor stage. The mucosal epithelium gives rise to adenomas, sessile serrated lesions, adenocarcinomas,goblet-cell adenocarcinomas, and mucinous neoplasms. Neuroendocrine neoplasms originate in neuroectodermal tissue. Adenomasof the appendix can usually be definitively treated by appendectomy. Mucinous neoplasms, depending on their tumorstage, may require additional cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemoperfusion (HIPEC). Adeno -carcinomas and goblet-cell adenocarcinomas can metastasize via the lymphatic vessels and the bloodstream and should thereforebe treated by oncological right hemicolectomy. Approximately 80% of neuroendocrine tumors are less than 1 cm in diameterwhen diagnosed and can therefore be adequately treated by appendectomy; right hemicolectomy is recommended if the patienthas risk factors for metastasis via the lymphatic vessels. Systemic chemotherapy has not been shown to be beneficial forappendiceal neoplasms in prospective, randomized trials; it is recommended for adenocarcinomas and goblet-cell adenocarcinomasof stage III or higher, in analogy to the treatment of colorectal carcinoma.

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